Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study

Mahi, Sarah-Louisa; Bahram, Siamak; Harambat, Jérôme; Allard, Lise; Merlin, Étienne; Belot, Alexandre; Ranchin, Bruno; Tenenbaum, Julie; Magnavacca, Marie; Haumesser, Lucile; Allain-Launay, Emma; Piètrement, Christine; Flodrops, Hugues; Ruin, Mahe; Dossier, Claire; Decramer, Stéphane; Ballot-Schmitt, Claire; Boyer, Olivia; Seuge, Laure; Ulinski, Tim; Zaloszyc, Ariane

doi:10.1007/s00467-022-05855-0

Cited by 10 publications

(8 citation statements)

References 37 publications

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“…With regards to organ involvement, our ndings were comparable to other prior studies (5,9,10). Within the GPA cohort, ENT involvement was more frequent.…”

Section: Discussionsupporting

confidence: 90%

“…Increasing rates of incidence of childhood AAV has been demonstrated (5,6), similar to observation from adult studies (7,8). Most of the existing literature on childhood onset AAV is derived from Europe and North America with predominantly white patients (5,9,10). Studies in adults with AAV have reported variability in the clinical phenotype of disease between various ethnic and racial backgrounds (11).…”

Section: They Include Granulomatosis With Polyangiitis (Gpa) Microsco...mentioning

confidence: 99%

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Childhood-Onset ANCA- Associated Vasculitis: Single center experience from Central California

Singh¹,

Sukumaran

2023

Preprint

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Background Childhood-onset ANCA-associated vasculitides (AAV) are rare and characterized by necrotizing inflammation and include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Pediatric data is scare and there have been no prior studies examining the characteristics of pediatric AAV in Central California, which has a Hispanic majority population.Methods We conducted a retrospective study of patients ≤18 years of age diagnosed with AAV between 2010 and 2021, in Central California. We analyzed demographic, clinical and laboratory characteristics, treatment, and initial outcomes.Results Of the 21 patients with AAV, 12 were categorized as MPA. Median age at diagnosis was 13.7 years in MPA cohort and 14 years in GPA. MPA cohort had significantly high proportion of females (92% versus 44%). Fifty seven percent of the cohort were racial/ethnic minority including Hispanics (n = 9), Asians (n = 2), and multiracial (n = 1) and 43% were white (n = 9). Hispanic patients were more frequently diagnosed with MPA (89%) and positive for myeloperoxidase antibody (100%). White patients more often were diagnosed with GPA (78%) and expressed PR-3 positivity (89%). Median duration of symptoms prior to diagnosis was 14 days in MPA cohort and 21 days in GPA cohort. Renal involvement was frequent (100% in MPA and 78% in GPA). GPA cohort had more frequent ENT involvement (89%). MPA cohort tended towards more severe disease with 67% requiring ICU admission and 50% requiring dialysis. Two deaths were reported in MPA cohort, related to Aspergillus pneumonia and pulmonary hemorrhage. In MPA cohort, 42% received cyclophosphamide in combination with steroids and 42% received Rituximab in combination with steroids. GPA patients received cyclophosphamide, either with steroids alone (78%) or in combination with steroids and Rituximab (22%).Conclusions Microscopic polyangiitis was the most frequent AAV subtype with female preponderance, shorter duration of symptoms at onset and higher proportion of racial/ ethnic minority patients. We observed more frequent MPO positivity in Hispanic children. Trends towards higher rates of ICU admission and dialysis was noted in MPA. Patients with MPA received Rituximab more frequently. Future prospective studies are needed to understand differences in childhood onset AAV between diverse racial-ethnic groups.

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“…With regards to organ involvement, our ndings were comparable to other prior studies (5,9,10). Within the GPA cohort, ENT involvement was more frequent.…”

Section: Discussionsupporting

confidence: 90%

Section: They Include Granulomatosis With Polyangiitis (Gpa) Microsco...mentioning

confidence: 99%

Childhood-Onset ANCA- Associated Vasculitis: Single center experience from Central California

Singh¹,

Sukumaran

2023

Preprint

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“…AAV have the potential to be life threatening and often are associated with marked morbidity and mortality. Unfortunately, most available evidence stems from studies in adults as there are only a few small studies done in the pediatric population [4,5 ▪ ,29 ▪▪ ,30 ▪▪ ]. Similarly to LN, the treatment approach is rapidly evolving as the pharmacologic options are growing, so new studies are constantly changing the way we approach the disease.…”

Section: Anti-neutrophil Cytoplasmic Antibody Associated Vasculitidesmentioning

confidence: 99%

“…Kidney involvement was the most common presentation of AAV at 78%, with 20% of patients requiring dialysis at presentation. Indeed, 20% of patients developed CKD stage 3–5 within 1 year of their AAV diagnosis [29 ▪▪ ]. Despite increased rituximab usage during the study period, AAV outcomes, or AAV kidney relapses, remained stable over time [29 ▪▪ ].…”

Section: Anti-neutrophil Cytoplasmic Antibody Associated Vasculitidesmentioning

confidence: 99%

Renal disease in pediatric rheumatology

Cody,

Brunner

2024

Current Opinion in Rheumatology

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Purpose of review This review will provide updates in the outcomes in the common rheumatologic diseases with kidney involvement. Covered are also advances in therapeutics for the use of pediatric rheumatologic diseases with kidney involvement, as well as the potential kidney complications from other rheumatologic diseases and their medications. Recent findings Two of the more common rheumatologic diseases with kidney involvement, lupus and vasculitis, continue to show inadequate response to initial therapy of renal disease and practice continues to be driven by results of adult studies. Summary There is a continued need for pediatric specific studies in rheumatologic diseases with kidney involvement as outcomes continue to be inadequate. Despite recently approved treatments for adults with rheumatic diseases and kidney involvement, therapeutic options in pediatrics remain limited, contributing to the overall morbidity and mortality.

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“…Even with modifications in the treatment regimens over the past several years, systemic outcome remains relatively poor with nearly half of patients (45%) experiencing one or more clinical relapses despite significant improvements in rate of remission at the end of the induction period (80.4%), despite substantial chronic kidney disease (54.8% at 1 year) and clinically significant adverse events (in 54%) leading to treatment modifications (typically requiring dose reduction of CYC or switching to RTX). As prospective studies are needed to assess the most appropriate therapeutic modality for individual children with AAV [71], the same can be said for cPACNS and its subtypes.…”

Section: Theme 10: a Comparison Of Small-vessel Childhood And Adult P...mentioning

confidence: 99%

Primary central nervous system vasculitis and headache: Ten themes

Younger

2023

Current Opinion in Neurology

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Purpose of review The primary central nervous system (CNS) vasculitides refers to clinicopathologic disorders that share the histopathology of inflammation of cerebral or spinal blood vessels. Unrecognized and therefore untreated, vasculitis of the CNS results in irreversible injury and disability making these disorders of paramount importance to clinicians. Recent findings Headache is an important clue to vasculitic involvement of CNS vessels. CNS vasculitis can be primary, in which only intracranial or spinal vessels are involved in the inflammatory process, or secondary to another known disorder with overlapping systemic involvement. The suspicion of vasculitis based on the history, clinical examination, and laboratory studies warrants prompt evaluation and treatment to prevent cerebral ischemia or infarction. Summary Primary CNS vasculitides can be diagnosed with certainty after intensive evaluation that includes tissue confirmation whenever possible. As in its systemic counterparts, clinicians must choose from among the available immune modulating, suppressive, and targeted immunotherapies to induce and maintain remission status and prevent relapse, tempered by anticipated medication adverse effects.

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Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study

Cited by 10 publications

References 37 publications

Childhood-Onset ANCA- Associated Vasculitis: Single center experience from Central California

Childhood-Onset ANCA- Associated Vasculitis: Single center experience from Central California

Renal disease in pediatric rheumatology

Primary central nervous system vasculitis and headache: Ten themes

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